What are your preferred lipid-lowering agents and target LDL reduction goal following initiation of therapy for patients with familial hyperlipidemia without underlying CAD? 

It is not easy to address this question without specifics of the 'familial hyperlipidemia," but I will give examples:

1. Monogenic familial hypercholesterolemia - since the LDL is very high from early childhood, we tend to treat adolescents with statins with a goal of a 50% reduction (20 or 40 rosuvastatin; 40-80 atorvastatin. If LDL is well over 200 untreated, ezetimibe would likely be added soon after. In adults over 21, never treated, statin and ezetimibe in rapid sequence with a goal of <100 mg/dl or <70 mg/dl depending on the presence of other risks - early CAD in family, diabetes, hypertension. I put Lp(a) in a separate category, and until we have drugs available (and they lower ASCVD), I try for LDL <50 mg/dl in FH with a Lp(a) above t...

I agree with Dr @Dr. First Last that familial hyperlipidemia is a term that is difficult to interpret without the actual lipid panel and family history data.

If the genetic condition is a primary severe hypercholesterolemia (monogenic FH or polygenic hypercholesterolemia), statins and nonstatins based on LDL-C goals (<100, <70 depending upon risks). PCSK9-based therapies are indicated for these conditions and should be offered and prescribed if goals not attained.

If the lipid disorder is a polygenic combined hyperlipidemia -what we used to refer to as familial combined hyperlipidemia- this condition is also very high ASCVD risk (with clues obtained from family history of premature CAD in several family members), and therefore statin and no...